Otological manifestations of granulomatosis with polyangiitis (GPA) are uncommon, reported in up to 35% of cases (1), and is very rarely the presenting complaint (2). A rare case is reported of a 56-year-old male who initially presented with a new right facial nerve palsy secondary to presumed chronic/recurrent otitis media. This was on the background of 3 months of right sided otalgia, hearing loss and recurrent perforation with otorrhoea. Following a CT petrous temporal bone demonstrating middle ear and mastoid effusion with a dehiscent nerve, a grommet was placed. While there was some initial improvement in facial nerve function from House Brackmann (HB) IV to HB II, this then subsequently worsened and persisted following conservative management with IV dexamethasone and topical ciprofloxacin-hydrocortisone drops. He subsequently underwent cortical mastoidectomy which demonstrated thickened inflamed granulation tissue in the middle ear and hyperostotic bone in the mastoid. An autoimmune screen was sent at this time while on steroids, which was negative. He re-presented while on a weaning dose of prednisolone with intermittent fullness, otorrhoea and fluctuating facial nerve function post-operatively. In the interim he also developed right sided hydronephrosis requiring cystoscopy and stent insertion for external retroperitoneal compression of right ureter. A repeat autoimmune screen then flagged positive with a high c-ANCA. A diagnosis of granulomatosis with polyangiitis was made and the patient started treatment with rituximab and prednisolone with stabilisation in hearing loss. References: 1: Greco A, Marinelli C, Fusconi M, Macri GF, Gallo A, De Virgilio A, Zambetti G, de Vincentiis M. Clinic manifestations in granulomatosis with polyangiitis. International journal of immunopathology and pharmacology. 2016 Jun;29(2):151-9 2: Mcdonald TJ, Deremee RA. Wegener's granulomatosis. The Laryngoscope. 1983 Feb;93(2):220-32.